Background
Transjugular intrahepatic portosystemic shunt (TIPS) is a well-established approach for the treatment of portal hypertension-related complications in patients with cirrhosis [1].
Patients with porto-sinusoidal vascular disorder (PSVD) can experience portal hypertension-related complications similar to patients with cirrhosis. Still, they deeply differ from these patients in terms of age, pathophysiology of the underlying liver disease and comorbidities. They usually present a preserved liver function and a higher incidence of splanchnic vein thrombosis that can hamper the TIPS creation [2].
While the indications and strategy for TIPS positioning in patients with cirrhosis are well defined by several guidelines [1; 3], there is a significant gap in knowledge regarding the management of portal hypertension-related complications with TIPS in patients with PSVD. Studies carried out so far in this context are mainly small case series, including patients defined as patients with non-cirrhotic portal hypertension rather than PSVD, reporting data related to TIPS positioned until 2015 when the definition of the vascular liver disorders, type of stentgraft used and clinical approach to treat these patients were completely different.
These observations were the basis for proposing this study, which aims to evaluate the technical success, early complications, long-term clinical outcomes in terms of further decompensation and/or recompensation and survival of adult patients with PSVD that required TIPS placement.
Study design and data collection
This is multicentric retrospective study. Patients with PSVD requiring TIPS positioning for portal hypertension-related complications until 31/12/2023 will be identified from participant centers. Data will be retrieved from the medical records of the included patients and will be pseudonymized. Follow-up will start at the time of TIPS positioning and will end on 31/12/2024.
The following data categories will be retrospectively collected by the local investigators and centralized for analysis by the principal investigators of this study:
- Demographics data: age, sex, BMI;
- Data related to liver histology showing signs of PSVD: description of the liver biopsy
- Medical history/risk factors for PSVD: myeloproliferative disorders, coagulation disorders, systemic or auto-immune comorbidities, prior drug exposure (potentially toxic), other concomitant causes of hepatopathy;
- Data related to portal hypertension: CT-scan (spleen size, porto-systemic shunts, portal vein diameter), endoscopic (esophageal and gastric varices and their size), non-invasive assessment (liver and spleen stiffness if available), clinical evidences;
- Data related to the management of portal hypertension before TIPS: NSBB, EVL, diuretics, albumin, lactulose, rifaximin, anticoagulation;
- Indication to TIPS positioning: variceal bleeding, refractory/recurrent ascites, portal vein thrombosis;
- Data related to pre-TIPS positioning work-up: lab tests, imaging (CT scan) and cardiological assessment;
- Data related to TIPS positioning: systemic hemodynamic before and after TIPS positioning, type of stentgraft used, stentgraft dilatation;
- Data related to early post TIPS complication: TIPS dysfunction, liver failure and/or cardiac failure post TIPS;
- Data related to long-term outcomes post TIPS positioning: incidence of rebleeding, ascites control, post-TIPS overt HE requiring hospitalization, recompensation, portal vein thrombosis (without TIPS dysfunction);
- Survival/liver transplant.